Congenital intrahepatic portosystemic shunt diagnosed during intrauterine life

Camila Vieira Bellettini , Rafaela Wagner , Aleocídio Sette Balzanelo , André Luis de Souza Andretta , Arthur Nascimento de Moura , Catia Carolina Fabris , Eduardo Maranhão Gubert
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引用次数: 4

Abstract

Objective

To report a patient with prenatal diagnosis of portosystemic shunt; a rare condition in humans.

Case description

17-Day-old female infant admitted for investigation of suspected diagnosis of portosystemic shunt, presumed in obstetric ultrasound. The hypothesis was confirmed after abdominal angiography and liver Doppler. Other tests such as echocardiography and electroencephalogram were performed to investigate possible co-morbidities or associated complications, and were normal. We chose conservative shunt treatment, as there were no disease-related complications and this was intrahepatic shunt, which could close spontaneously by the age of 2 years.

Comments

Portosystemic shunt can lead to various complications such as hepatic encephalopathy, hypergalactosemia, liver tumors, and hepatopulmonary syndrome. Most diagnoses are done after one month of age, after such complications occur. The prenatal diagnosis of this patient provided greater security for the clinical picture management, as well as regular monitoring, which allows the anticipation of possible complications and perform interventional procedures when needed.

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先天性肝内门系统分流诊断在宫内生活
目的报告1例产前诊断为门静脉系统分流的患者;一种罕见的人类疾病。病例描述:17天大的女婴因产科超声诊断疑似门系统分流而入院调查。经腹部血管造影和肝脏多普勒检查证实了这一假设。其他检查如超声心动图和脑电图进行调查可能的合并症或相关并发症,并正常。我们选择了保守的分流治疗,因为没有疾病相关的并发症,而且这是肝内分流,可以在2岁时自动关闭。运动系统分流可导致各种并发症,如肝性脑病、高半乳糖血症、肝脏肿瘤和肝肺综合征。大多数诊断是在一个月大的时候进行的,在这些并发症发生之后。该患者的产前诊断为临床图像管理和定期监测提供了更大的安全性,从而可以预测可能的并发症并在需要时进行介入治疗。
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